Congenital oculomotor palsy: associated neurological and ophthalmological findings

Congenital fourth and sixth nerve palsies are rarely associated with other evidence of neurological abnormality, but there have been conflicting reports in the literature on the associations of congenital third nerve palsy. In order to clarify the situation we report a series of 14 consecutive cases presenting to a paediatric tertiary referral service over the last 12 years. In this series of children, 5 had associated neurological abnormalities, lending support to the view that congenital third nerve palsy is commonly a manifestation of widespread neurological damage. We also describe for the first time a phenomenon of digital lid elevation to allow fixation with the affected eye. Two children demonstrated this phenomenon and in each case the accompanying neurological defect was profound. The frequency and severity of associated deficits is analysed, and the mechanism of fixation with the affected eye is discussed.